Friday, 27 February 2015

The
year was 2002, I was still 18 and my mom wanted to go to Colorado
because a pseudo-sister was having her first child and wanted my mom
there. Not being the one
to let my mom travel cross country in the car by herself, I opted to go
with. It was going to be great, I was going to be able to go back to
where I grew up, see friends/family and most importantly enjoy the
scenery I have come to miss more and more each day.
The road trip started out like any typical road trip loading up the
car, getting fuel and hitting the road. We stopped in Hesperia to get
food at In-N-Out and then continued on. It was night time (because no
one wants to drive across the Mojave Desert during
the day) and I was slated to drive at some point. My mom wanted to
drive first, until she got tired, and then she was going to let me take
over—or at least that’s what we thought.

After
eating and the getting on the road, I fell asleep to make sure I was
well rested to take on the second leg of the journey. In Flagstaff,
Arizona we stopped for
fuel and I proceeded to get sick. Maybe it was bad food… no one really
knows—actually, they do. I was going into heart failure but didn’t know
it. We continued on the road trip, but I still was sleeping a lot.
Unfortunately, my mother had to drive the whole
trip by herself because I was just that tired. Something clearly wasn’t
right, but maybe it was the food. Ok, not really but we didn’t know
better at the time.

Upon
arrival to Colorado Springs, I had another episode where I made my
mother pull over so I could proceed to get sick outside of the car.
Walking around at altitude
was difficult, I couldn’t breathe well and it felt like I had an
elephant tap-dancing on my chest in stilettos. Walking into a restaurant
was quite interesting. Well, I didn’t actually make it into the
restaurant, 10 feet from the car the elephant started
his Irish jig, and 30 seconds later I was blue and on the ground. I
woke up shortly afterward without much recollection of what had
happened. However, I had previously had an episode like this four months
prior while dropping off a portly cat at my former
bosses house before I moved to California. Finally, my mother could see
what the black-out episodes were really like. Still, we thought nothing
of it. Well, my mom thought something of it, I was oblivious to it
probably because of the invincibility complex
that most 18 year old males have at that point in their life. After
eating a late dinner, I felt a little better, perhaps it was the
company, regardless sinister things were still taking place much to our
unsuspecting wonder.

Eventually
we made it back to the hotel, and walking from the parking lot to the
room was a struggle. Horton the Elephant seemed to want to crush my
heart with his
size huge stilettos and bad dancing. Though out the night I was running
to the bathroom, throwing up over and over. Another case of food
poisoning? No. A stomach flu? No. The next morning I took a bath to
clean up from the night of sweating and puking my guts
out. After getting out of the tub I sat on the toilette. Next thing I
knew I woke up under the vanity, my head hurting and my chest pounding. I
proceeded to get dressed and sat at the foot of my mom’s bed. Not 5
minutes later she walked in with Michelle, my
long time babysitter though elementary and middle school. “Mom, it
happened again,” I said. I think all the color went out of her face at
that point, and Michelle stepped in and said, “You’re going to the
hospital whether I have to drag you kicking and screaming
or not.” While still reluctant, I went with it. We stopped by Shrap
Metal to get retainers for my piercings at the time (I mean who want’s
lightning rods in their body if you code?). Off to the hospital we went.

Upon
arrival at the hospital, I was taken into triage. The nurse put on a
pulseox and took my blood pressure. While my blood pressure was normal,
my oxygen saturations
were low, but more importantly my pulse was through the roof. She
thought the machine was broken… she tried the other hand… same result…
she took it by hand… same result. Next thing I know I was being whisked
to the cardiac area of the emergency room. There
was a slew of doctors and nurses around me. All I can recall is an echo
and chest x-ray at the time. I still didn’t want to stay, and I told
the doctor the only way he was keeping me was if I had a hot nurse. Of
course, the doctor delivered and my ICU nurse
was gorgeous.

The
next week was pretty intense, I think I had every test known to man run
on me including the infamous right heart catheterization. The
pulmonologist and cardiologist
taking care of my case came in and said, “We are pretty sure you have
pulmonary hypertension, but we’re not 100% sure. We need to send you up
to Denver where they have pulmonary hypertension specialists to evaluate
you further.” On my birthday, October 18th,
2002 I was transferred to St. Joseph’s Hospital in Denver, Colorado.
Dr.’s Lang and Kenard wasted no time and completed the work up for
pulmonary hypertension. After reviewing everything Dr. Lang came in and
said, “Sean you have Pulmonary Hypertension… right
now there are two medications, Flolan and Tracleer. Tracleer is new and
with as advanced as your case is you probably won’t get any benefit out
of it before the inevitable. Flolan is the Gold standard and would
probably be your best bet. If you don’t do any
treatment, you’re looking at maybe 2 years.”

Yeah, let’s just say my 19th
birthday present from a doctor was what seemed like a death sentence.
Fortunately, St. Joe’s was a teaching hospital. Every
floor had tons of laptops. I was scared, but very compliant so the
nurse brought me in a laptop. I spent most of the night researching PH
on my own. Mind you, in 2002 there weren’t tons of resources that were
easily accessible. After spending the rest of the
night soul searching and trying to decide what I wanted to do I fell
asleep. My mom arrived back at the hospital early that morning, and the
doctor stopped in. I told Dr. Lang know that I’d go with the Flolan,
after all, who really wants to die at 21—and that’s
what he pretty much gave me. I was slated to have a central line put in
and the rest is history.

After
getting back to California about two weeks after this whole ordeal took
place I met with my current pulmonologist. He told me at that point I
wasn’t likely ever
going to work or go to school. The same sentiment was resonated by the
PH specialist I saw in Los Angeles. However, as I began increasing on
the Flolan I began feeling better. My current pulmonologist never
mentioned the work or school thing again. I told
him I was going to go back to school and he supported me. Now I am a
second year Medical Student at Western University of Health Sciences
College of Osteopathic Medicine of the Pacific in Pomona, California.
I’ve worked, I’ve loved, and I’ve lost but I couldn’t
be more blessed.

The
Pulmonary Hypertension Association contacted me in early 2009 about
things they could do to make PHA better for young adults. In December of
2009, I was asked to
join the Young Adult Advisory Board in which Generation Hope arose with
special thanks to Colleen B., Britt R., Lindsay N. and myself. Shortly
after that, I was asked to become a PHA PH Patient Mentor. In 2012,
while getting my Masters of Science in Medical
Sciences, I got a wild idea to come up with a cartoon series for
children recently diagnosed with PH and so Lips Painted Blue (www.lipspaintedblue.com) was born. Needless to say, PH has shown me everything that
I can do.

Mind
you, I still have bad days occasionally, especially when modifying
medications, life couldn’t be better. I will take the first part of my
boards this summer and
then finally head out to rotations to do good with actual patients. I
will be able to take my experiences as a patient and relate to other
patients to ensure they get compassionate and comprehensive care. My
journey with PH may have begun back in 2002, but
I look at each day as a new beginning. Every day gives me the
opportunity to learn something new about myself. I couldn’t love life
any more than I do, and continue to preserver. The good will always
outweigh the bad, and the resources that are available for
individuals are limitless. Through opportunities like the Tom Lantos
Innovations in Community Service Award from PHA, if a resource doesn’t
exist you can let your creative juices flow and create it.

My
final remarks are something that I still believe to this day. Even
though you have PH, it doesn’t have you. You should never give
up, never surrender and most definitely live your life to the fullest.
If you have any questions you can feel free to contact me at
sean.wyman@gmail.com

Wednesday, 25 February 2015

When you first found
out about Reinee's diagnosis how did you feel? Has that feeling, and how you
view PH changed throughout the years? Is there anything that scared you
about the diagnosis at first that has changed?

Mike:Reinee first told me about her condition only
after a month or so of dating.It was a
very intimate setting – lights off and blanket over our heads – in the living
room of my apartment, just us two hanging out.She softly spoke and told me about her condition.At first, I didn’t comprehend the severity of
PH, that there was no cure and that it would progressively get worse.But, honestly I didn’t care how this would
impact my life because I already saw her being a part of mine.I fell in love with this girl.That night, I made it a point that I would
support Reinee but not treat her any differently than I did prior to
knowing.Since then I never looked back
and never strayed away from the promises I’ve made to her that night.Not once have I ever felt obligated to stay
with her or take care of her during the down times because I was willingly glad
to and I just wanted to be with her… All of her.

I guess the
only thing that scared me about her condition was knowing that it could get
worse to the point where I’d lose her.I
will admit that there were some close calls but ever since we got married about
a year ago, our bond has never been stronger and both of us are the healthiest
we’ve ever been.

Are there any activities that you and
Reinee do to together? For example, vacation once a year, walking the dog for
30 minutes after dinner, cooking, watching movies etc

Mike:I’m very proud of Reinee and all the things
that we’ve been able to accomplish just this year alone.Ever since the installment of her new
medication (Tyvaso), it has given her the ability to become more active and
endure more strenuous activities.I’ve
literally witnessed her from being out of breath after walking up a flight of
stairs to her first time snowboarding with me just this past weekend!Now snowboarding can be very tiring even for
me at times but Reinee, although cautious, arose to the occasion and attempted
to ride down bunny slopes like she had no PH at all.At the end of the day, she was S-curving the
slopes and was hardly falling down.

It also
brings me joy knowing that I’ve been able to experience all these “firsts” with
her.Earlier this year, we were
vacationing in Oahu and I finally persuaded her to hike up Diamond Head with
me.We hiked up that ~1 mile crater and
were able to share the miraculous views from the top.At that moment, I really felt that this was a
milestone for Reinee and it made her realize that if she was able to do this,
that there’s no telling what else she has the potential of accomplishing.Later on that trip we went paddle boarding
and further into the year we also tried indoor rock climbing for the first time
too.Again, another feeling of
self-fulfillment as I saw Reinee ringing the bell atop of each climb she made.

Is there anything that
you do to help Reinee with her condition? For example, carrying laundry up the
stairs.

Mike: Simply put, I’ll do anything and everything in
my power to help her with her condition.I’ll do anything from helping her set up her medication to carrying all
the groceries/laundry from point A to B.Although Reinee does a great job of holding her own which I greatly
appreciate, I will never hesitate to help her with whatever she needs.

What impresses you the most about
Reinee? What do you admire about her the most? Is there anything about her
personality that you feel has helped her thrive?

Mike: I love her fight and
unwillingness to give up on herself and our future.We take it a day at a time but definitely
have our sights on raising a family of our own.This is our goal in life and although she cannot bear a child for
herself we’re looking into all options and it has led to the path of surrogacy.Wish us good luck!

I admire
the fact that Reinee continues to live her life regularly, meaning she carries
herself as if she has no condition at all.At the same time, Reinee knows her limits and knows when to slow to down
if her PH gets the best of her, but this is always short lived.Reinee can bounce back and continue to act as
if nothing is wrong.Her resilience is
impeccable and is something that motivates me to be a better person for myself
and for each other.

Reinee’s personality
is fun-loving and upbeat.She’s a
pleasure to be with and it’s always a laugh fest when we’re with each
other.We’re very random too about the
things we talk about or the things we do like singing/rapping out loud while
driving in the car or spontaneous tickle fights in bed before falling
asleep.Reinee knows I’m very ticklish
and takes advantage of it all the time when she wants something.She has this power over me and without fail
can always make me feel better when I’m down and keeps me in check when I need
to be! Lol

Is there any advice
you would give to other people who are in a long-term relationship with someone
with PH, or anyone who is considering marriage with someone with PH?Mike: Like any relationship
everyone has their own “deal” that they bring to the table, but you as the
significant other must be able to love that person for both the good and the
bad.When Reinee and I were taking
marriage prep classes, the deacon told us that love is unconditional and you
must accept each other as whole, especially for their faults.You’re not just marrying the person you fell
in love with, but also the person that gets on your nerves and irritates the
heck out of you sometimes.This message
solidified how I felt for Reinee and that I would love her despite any deal she
had.

But maybe
it was easier for me to be with Reinee because she appeared to be a healthy
person when I first met her.And maybe
it was also for the fact of dating an older woman that attracted me to her
more, but whatever it was I knew she was someone that I could relate to and
most importantly have fun with.Reinee
was honest with me about her PH and upfront about the possibilities that could
happen throughout our relationship.But
until now, we’ve weathered the storms, overcome every obstacle, and continue to
stride forward with gusto because nothing is going to hold us back from living
our lives to the fullest and enjoying our family to we’re old and grey.

Friday, 20 February 2015

On October 1, 2012 my life changed
forever. This should have been the happiest time of my life. I had just celebrated my 3 year old’s
birthday and had a 6 month old. My life should have been at its peak, but that all changed on that
day.

For several weeks prior to that fateful day, I had been having what I thought
was “panic attacks”. I found myself completely out of breath. I
thought that maybe I was suffering some sort of social anxiety because these attacks coming on
mostly out in public. Slowly but surely they started happening at home. I was slowly unable to
even walk 10 feet without feeling air hunger. I couldn’t get enough air in my lungs and
was starting to get scared.

On the morning of October 1st my oldest
son had a speech appointment just like he had every single week. I got up to go to the
bathroom that morning and was so out of breath and was having horrible palpitations. This was
starting to get very scary and annoying all at the same time. I made it back to my bed and called
my Dad. I was in tears when I was on the phone and told him, “I just can’t do this anymore. I
need to seek help for this anxiety.” I then called my son’s speech teacher and told her there was no
way we were going to make it because I wasn’t feeling well.

After calling my kids were hungry and I
hadn’t been to the store due to my “social anxiety”. So, I loaded up my almost 6 month old and 3 year
old into the car. I started walking to my front door to lock it and BOOM. My sister found me
laying on my front steps turning blue and lifeless. My husband began to do CPR and they called
911.

The next thing I know I woke up in the
back of an ambulance and thinking, “this HAS to be a dream. Why am I here?” I woke up and
thought that I had a stroke because I had post partum REGULAR hypertension. I tried moving my
limbs and figured that I in fact didn’t have a stroke. I was admitted overnight to the hospital
and they ran every.single.test known to man. I was released the very next day with a
diagnosis of “anxiety”. I was told that I needed to seek some counseling and get on some Xanex or antidepressant
ASAP.

My cardiologist wanted me to follow up
with him the following day. I went in for a stress test and ECHO. The ECHO showed right ventricle
enlargement. I continued to the stress test which I only made it through the first minute and
a half before feeling like I was going to pass out and throw up. The cardiologist rushed in and
sent me to get a CT of my chest ASAP. She thought that I was having a Pulmonary Embolism.

The CT came back that I did in fact have
right ventricle enlargement that was consistant with Pulmonary Hypertension. My doctor told me
NOT to go home and look up Pulmonary Hypertension. He said he would do a heart
catherization to find out for sure what my pressures were. At this moment I didn’t think
anything of it. I heard the word “hypertension” and thought, “oh, great, more medication so I don’t
have a stroke.”

Of course the first thing I did when I
went home was got on my computer and Googled,“Pulmonary Hypertension”. The first thing
that popped up was the mortality rates. I worked myself into a real panic and thought there
is NO WAY I have this disease. I am a healthy 27 year old who just had a baby. I have 2
kids that I have to care for and this isn’t happening to me, but it was.

Not only did I have this horrible and
deadly progressive disease but now I was being told by every doctor that my dreams of having
another baby were dead in the water. I would never be able to carry another baby due to the high
mortality rates. This just added to my depression. I watched as my friends added to their
families. I just began to sink deeper into depression.

In mid November I finally had my right heart catherization that showed my mean
pressures were 65. I was diagnosed with severe pulmonary
arterial hypertension. I spent weeks searching the internet for anything that I could find
about this disease. I joined multiple support groups and researched and researched. I researched
myself into a depression. I had this impending feel of doom. I was just waiting to have another
attack and be laying lifeless on my front porch, except this time no one would find me.

In January of 2013 I was put on all oral
medications. The handful of meds I was prescribed was; Letairis, Adcirca, Warfarin, and Lasix. I
hated to take all these meds since I had never had to before but I know that they were the key
to saving my life. So, I took them and hated every second of it. About 3 months later I had
an ECHO follow up just to estimated my pulmonary pressures. At that time my pressures had
gone from 65 to 35. My doctors were ecstatic to see such a difference. I think one of my
cardiologist even cried out, “it’s a miracle!”

After having that ECHO my hope escalated!
I became empowered by my sickness instead of feeling depressed about it all the time. I
got a new lease on life. I decided that I will not let this disease define me. In fact, I will let it
encourage me. In April 2013, I opened my own business with my Mom. It was something that I had
always wanted to due but had pushed it to the back burner.

I continued my medications and kept on
with my “New Normal”. No, I was never going to be the old Amanda I was going to be the PHighter
Amanda now. It was battle time and I was ready to kick some butt. No more feeling sorry for
myself and being down in the dumps. I may have PH but that is not who I am it is what I have.

In May 2014 I had another routine ECHO to
check on my pressures. My pressures showed that my right ventricle was no longer enlarged
and my pressures were at 29! I knew that I was kicking this diseases butt. I did what I
wanted to do when I wanted to do it and didn’t let anything stop me. If I wanted to go run around and
chase my kids at the park I could. I continued on with my business and made a cross country move
with my family.

Finally, in December 2014 I had another
routine ECHO since I had switched doctors. She also exclaimed, “oh this is amazing, this is
soooo amazing!” My pressures were at 13 and my heart was doing great! She was amazed at the
progress I had made in just 2 short years.

Currently, I continue to operate my own
business, play with my kids when I want to, walk my dogs, ride bikes, clean my house for
hours, and most importantly my husband and I will be starting IVF soon to have our 3rd baby via
surrogacy. I am so excited about my life. PAH has become much less of a thought these days.
The medications remind me that I do have a disease but if I didn’t have those I
wouldn’t even think about my PAH.

A positive outlook on this disease has
helped me heal tremendously. Many of the support groups I have joined have also helped me
stay positive. Surround yourself with positive thoughts. I don’t want to get preachy but
my motto this entire time has been, “God gives his toughest battles to His strongest soldiers.”

Friday, 13 February 2015

Before I got PH I had a very active lifestyle, running, sailing,
caving, climbing, hiking up mountains, and my passion, horse-riding. All my life I owned, rode or worked with horses. I would think nothing
of biking 8 miles to work (running a Riding For the Disabled Yard) and
back, and then going for a 5 mile run in the evenings. I guess i was a
bit of an adrenaline junkie, competing cross country and show-jumping
with the horses…..and often going to theme parks to ride all the scary
roller coasters.

During the winter of 2009 we
had a lot of snow, and one evening my car got snowed in at the stables.
I ran the four miles home in the snow, getting pretty cold and wet. A
few days later I got pleurisy. I never really recovered from that. When all the snow had cleared I tried biking to work again and found it
incredibly hard. I tried to go out for a run, but found I was out of
breath after a few metres. I knew something was very wrong, but the
doctor assured me it was down to the pleurisy and I had to get fit
slowly again.

I spent the whole of 2010 trying
to do just that. I walked everywhere, but still couldn’t run. Going up
hills I used to run up I had to stop to catch my breath. I found
working the horses really hard work, and I kept getting a weird
tingling feeling down my arms and into my wrists. My hands and feet
were numb even in the summer!

Early in 2011 I
seemed to be getting worse and I went back to the doctor, who thought I
might have asthma and gave me an inhaler. I pushed myself so hard to
get fit I don’t know how I didn't kill myself instead. A few months
into 2011 I was at the doctors for a general check up and she asked how
my breathing was. As it was no better she sent me for a x-ray.

A
few days later I had a phone call from an alarmed doctor, who told me I
had an enlarged heart on the right hand side. I was told to be very
careful and was referred to hospital. After a series of tests one thing
after another was ruled out until finally 2 days before my 45th
birthday the consultant said he thought I had PH and was referring me to
a specialist hospital, the famous Papworth. In the mean time I
googled PH as we all do and was thrown into a panic after reading I only
had two years left to live!

I lost my job as I
was deemed too unfit, and I put my own pony out on loan. At that
point I couldn't even climb the stairs without stopping. Papworth
initially classified me as a WHO class 2/3 and started me on Sildenafil
and Warfarin. They helped a bit but I sunk into depression.

Without
the horses I felt like my right arm had been cut off. I found it hard
work just walking the dog round to the shop, a mere 1/4 mile away. I
was even jealous of runners. I mourned the loss of my lifestyle. I
applied for several jobs without success, and was in a really black
place.

Finally in April 2012 I got a job at
Tesco on the checkouts. Papworth also started me on Bosentan , and
those two things were the beginning of the turnaround. Slowly I started
to find I could do more, walk further and felt stronger. I discovered
the Facebook PH group and was amazed to find people that have lived with
PH for 20 years. I began to feel ‘normal.’ again.

In
April 2013 I got a job in my home town in the ice cream shop, which is
physically harder than Tesco, but again the more i did the better I
felt. I also got a second job in the evenings, waitressing in a bar,
and walked everywhere. In my 6 minute walk tests I could do nearly
600m. I almost forgot I had anything wrong.

This
year I have had a couple of set backs as I was diagnosed with Breast
Cancer and the local hospital refused to operate due to the risks of the anesthetic. Lucky for me really as I was referred to the brilliant
training hospital Addenbrookes only 10 miles from Papworth . I spent a
couple of days at Papworth having all the tests again, and was delighted
to discover the pressures in my lungs have lowered and I am now a Who
class 1. I sailed through local anaesthetic lumpectomy and removal of 3
lymph nodes, making history into the bargain, and the following 6 weeks
of radiotherapy. Unfortunately that caused pneumonia in October and I
ended up in intensive care, which has reminded me, that I do actually
have a lung condition and to slow down a little bit!!

Back
in the summer I met someone local who keeps horses and started helping
her out. To my delight I found myself back in the saddle, and not out
of breath! I am also trying to start a dog walking business, although I
only have one customer at the moment, I find it no problem striding
around the countryside, doing 3 miles or so. I firmly believe the
exercise helps the condition. So although at the beginning it felt as
though PH had stolen my life, little by little I have stolen it back
again!

Friday, 6 February 2015

In the PH community, I often hear people being asked what
their life was like before diagnosis. I've been asked this myself many
times over the years. The thing is, my life before I was diagnosed with
PH was the same as my life is now. I don't know what it's like to breath
normally. I don't have a clue what going from an active lifestyle to a
PH lifestyle is all about. That is because PH has been my entire life.
It's all I've known. I actually thought, as a kid, that it was normal to
take hours to recover from running around with your siblings and
friends. I thought everyone took naps when they were exhausted! And
although everyone around me, family included, always told me I looked
purple, to me that was just my thing!

I was
diagnosed when I was 9 months old, but technically, I should have been
diagnosed months earlier. My mom brought me to a free clinic to get my
first set of immunization shots, and a doctor there thought something
was up with my heart. My mom took me to the pediatrician, who didn't
think anything was wrong. So at the same free clinic for my 2nd set of
shots, the same doctor who saw me the first time told my mom that
something was seriously wrong with me, because I looked blue. My parents
ended up taking me to a children's hospital, and after many tests, they
discovered I had a pretty bad congenital heart defect and pulmonary
arterial hypertention. At that time (1975), the doctors couldn't do
anything. It was too late for surgery to correct the holes in my heart,
and there wasn't anything to treat PAH. My parents were told I might not
make it to my first birthday, or I may possibly live til I'm 50. They
weren't given much hope! But here I am, 39 years later, and I'm still
living to share my story!

First Airplane Trip

I grew up with a lot
of "cannots." I cannot participate in sports. I cannnot take gym class. I
cannot go on rides that'll get your heart rate up too much. I cannot
get pregnant. And so I lived my life following the "cannots." I often
wonder if that really was a good thing or a bad thing, but I guess since
I followed those rules pretty closely, and I'm still here, it was a
good thing? I followed my grade school dream of becoming a teacher,
getting my Bachelor's and eventually ending up in a preschool classroom.
I loved it! But after only a few years, those preschooler germs started
loving me. Too much. I was on my third major respiratory illness during
my third year of teaching when my primary doctor came into the exam
room with tears in her eyes and told me I had to quit my job. She wrote
me a note that I took to my director right after the appointment. I was
24 at the time, and never even thought about quitting a career at that
age. Who would??

A few years later after my PAH
symptoms continued to get worse, I ended up going to the Cleveland
Clinic in Ohio. I was referred by my doctor to go for a transplant
evaluation. At the time, the shortness of breath started happening more
frequently (just getting dressed in the morning was becoming a 30 minute
event), and an elephant had permanently moved in on top of my chest.
That was one symptom I didn't have growing up, and it was not a symptom I
particularly enjoyed! Being sent to Cleveland was very scary.
Transplant was even scarier!! But my first visit, I was sent back home
after so many tests with instructions to start taking Coumadin, a blood
thinner, and to wear oxygen all the time. After several more visits to
Cleveland in the following months, I was put on Tracleer. I had no idea
what it was, or exactly what it would do. All I knew was that I surely
hoped it would help my elephant to move on, and that I would start
feeling better!!

My first shipment of Tracleer
brought me not only the first medicine I'd ever try for PAH, but it also
brought me into the world of the pulmonary hypertension community. I
grew up knowing I had PH, but it was something that was never focused
on. My heart condition was the central star all those years. Once I
found the Pulmonary Hypertension Association website, my knowledge of PH
started to expand. But what really hit me?? THERE WERE OTHERS LIKE
ME!!!! I seriously spent a week reading the message boards and crying,
because there were people I could relate to and totally understand!! It
was like finding a miracle!

Tracleer got that
stupid elephant to move out several months after being on it, and I also
didn't feel as short of breath doing certain things like I was always
used to feeling. It was a pretty awesome feeling for a few years, until I
felt like I needed to add another medication to the mix. Revatio was
added, and the combination of the two is what I am still on today.
Pulmonary rehabilitation was something I found out about from phriends
(friends who have PH), and after inquiring about it, I started going
twice a week at one of my local hospitals. It's been a little over six
years since I've started pulmonary rehab, and I truly believe it's a
part of what's been keeping my PAH stable in the last several years.
Kinda hard to believe I went from no gym or sports as a kid/teen to
working out a couple times a week at least! No, I am not running
marathons or doing heavy weightlifting, but that is ok! Exercising at
any capacity is beneficial, even if I can only do it a little at a time!

So
after my PAH seemed to be stable for awhile, the next part of the
equation was....what am I going to do with my life?? I couldn't teach. I
knew I'd never go back to that. But I didn't know what else to do. Some
days I had more energy than others. I'd have one day where I wish I
could be at a job, to several days when I was thanking my lucky stars
that I didn't have one. Then one day I got a call that started the ball
rolling for me towards a direction I never thought about. I was asked if
I'd consider starting a support group for PH patients in my area. At
first, I was pretty terrified. I could handle being around kids, but to
lead a group for adults??? I guess you could say that my shy background
made the whole prospect a bit daunting for me! I really had to think
about it. So, that's what I did. I spent a couple weeks thinking and
praying and talking to my loved ones about the idea. I finally decided
to go ahead! Yes, I was incredibly nervous about it, but I felt it was
something I just HAD to do. How many years did I live with this disease
by myself?? I just knew I had to at least give others the chance to not
feel so alone after their diagnosis!

In the 10
years since starting a support group in the Niagara Falls/Buffalo, NY
area, I have really become involved with helping PH patients. I've had
so many meetings, a large variety of topics and speakers, and phriends I
care about very much. I have also become a big part of the online
community for PHers, by being a PHA mentor, a chat leader, a blogger,
and just a presence on Facebook. I had always wanted to teach in a
classroom, but it took me awhile to realize that a "classroom" didn't
have to be a place with 4 walls. It could be anywhere! I have even
educated complete strangers during shopping trips about PH! And, in the
10 years since starting my support group, I have also learned to not be
so shy with people. Sure, I can keep quiet sometimes, but more often
than not, I put myself out there and let others know about this awful
illness. Awareness is the key to so many answers, and I never know when
one day I will talk to someone and they tell me they have PH, too!

My
life with PAH is something I cannot change, and honestly, I think it
has helped shape me to be the person I am today. Do I have days when I
am angry about having it? Of course! I allow myself to have those angry
days, or days of self-pity, but not for long. What good does that do me?
I have learned to deal with this disease, and to cope the best I know
how, and I have learned to help others going through the same thing. I
have also not let PAH BE my entire life. I enjoy things like reading,
painting glass, making jewelry, spending time with family and friends.
In the last few years I have allowed myself to take on challenges I
never thought I could do, because I always had that "cannnot" attitude
with me. So I've gone on hikes, I've been on a jet ski, I took my first
ever trip on an airplane! There are so many more things I've let myself
try, even if I may not be able to do them again. I try my best not to
let PAH take over my life, because if I did, I can't honestly say that
I'd be living to the best of my ability!

Monday, 2 February 2015

So often people spend years searching for that
thing that inspires there life and gives them purpose. I am lucky because I
found my inspiration early in life and get to reflect on it daily. It is an
incredible feeling to have that kind of inspiration day in and day out. I am
going to pause here and give you a little background before I go any further…

My name is Josh and my wife’s name is Nicole, and
I was asked to share with you a little about our story. Nicole and I met in
college and it didn’t take long for us to realize that we were in love and
meant for each other. We got married May 13th, 2006. In a three week
period of time I graduated college, got married to the woman of my dreams, and
started a new job with a Fortune 500 company that had endless opportunity. Life
couldn’t be better. I felt like nothing could slow us down.

About two months after we got married, Nicole
started having recurring episodes where she would pass out if she pushed herself
to hard physically.At first, the
episodes were not severe and according to her doctor at the time, were
something Nicole would just have to sort of “manage” by adjusting our lifestyle
and taking medications.

Prior to us getting married Nicole had some very
minor symptoms but nothing like this and doctors never seemed to be worried. One
doctor told her the symptoms were all in her head and that nothing was wrong
with her. It’s like I knew something was wrong but since the doctors didn’t
seem too worried about it, I was hopeful it wouldn’t get to bad. I just knew
that I would be there to help her and we would get through it, whatever it was.

In a short period of time the symptoms worsened
and her passing out turned into violent convulsions. One night she had an
episode that was so severe she stopped breathing.I started to give here CPR and called
911.I had no clue what I was doing but
somehow she came to and started breathing again. I am not sure there is a way
to explain the fear I felt at that moment. I thought she was going to die in my
arms right there. Lucky for us God was on our side that night.

The paramedics arrived shortly after I got her to
start breathing again. We ultimately ended up in a hospital in downtown Kansas
City where she was admitted and moved to the critical care unit. Doctor’s ran several
tests to try to figure out why she was having these episodes.She continued to have them in the hospital
and actually coded two more times and was revived by the medical staff. I felt
like life was falling apart and I was losing the most important thing in the
world. After a few days of being admitted she was diagnosed with Primary
Pulmonary Hypertension (PH). I remember feeling confused and wondering what
that really meant.Is it treatable? Is
she going to be ok?

The doctor explained the disease was a chronic
lung disease that causes the heart to fail and that that the average life
expectancy (at the time) after being diagnosed was 3-5 years and with a heart
and lung transplant it could extend from there. He also noted there were
treatment options but very few patients actually get better after starting
treatment. For most patients treatment only slowed the progression of the
disease.

Looking back I remember thinking heart and lung
transplant… really?How is this
happening?She is so young and really
took care of herself. Why Nicole, why us? Life is not fair. Anger, pain, denial
are all feelings that came to me over the next few days. There is no way to
describe the experience we went through. This was the bottom.

Well let me explain how things can change.

While life at that moment seemed lost, God had a
plan for us. I have never seen anything like it in my life. Nicole was
determined to get better so we could live a full life together and it was like
God was with her every step of the way.There is a power in positive thinking because Nicole always finds a way
to bring out the positive side of every situation. Each day Nicole seemed to
get better. Am I saying it was easy no, there were days of struggle, sickness,
and symptoms but somehow we got past it.

Over the next few years, life slowly returned to
“normal”.By “normal” I mean
exceptional. Nicole got better and better.She went from being on constant IV infused medication to an inhaler
(nebulizer).Growing up in Pensacola,
Nicole dreamed of the day she could swim in the water again and guess what… she
does every summer now! Not only is she physically better, we have grown as a
couple and as a family. We have two beautiful children now (both adopted since
we couldn’t biologically have children). They are amazing and the light of our
life. If you think that PH is the end and you can’t get better, stop and know
that it’s not true. Sure there are days
she doesn’t feel the greatest and yes she is still limited by physical
activities but in the grand scheme of things, she can do more than she can’t.

We are an example of how being positive will carry
you through. Pretty sure it’s evident by now but going back to where I started,
Nicole is what inspires my life and fills me with purpose. Sometimes people
tell me I am a good person because I have taken care of her but what they don’t
know is that it’s the exact opposite.Without her I would be lost. She is an amazing mother and wife that is
the driving force behind our family.

So for those of you that have someone you love
going through this be positive and uplifting.Your spouse/significant other needs your support and deserves it.It can be tough but I promise you it means so
much to them just for you to be at their side smiling and telling them you love
them. Live in the day and know that God’s plan will take you where you need to
go. It is so important to enjoy every day of the journey, even the tough ones.Let the little things
life throws at you go as it’s not worth it to stress over. Who knows, you could
end up like me with all your hopes and dreams coming true.